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Von Willebrand disease

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https://www.readbyqxmd.com/read/28726980/interdependence-between-osteoprotegerin-and-active-von-willebrand-factor-in-long-term-cardiovascular-mortality-prediction-in-patients-undergoing-percutaneous-coronary-intervention
#1
Jolanta Siller-Matula, Irene M Lang, Christian Schoergenhofer, Mark Roest, Bernd Jilma
The interdependence of the predictive accuracy of serum osteoprotegerin (OPG) and von Willebrand factor (vWF) levels for long-term cardiovascular outcomes has not been investigated so far. This was a prospective observational cohort study in 361 patients with coronary artery disease undergoing percutaneous coronary intervention (PCI). Baseline levels of OPG, vWF, active vWF (act vWF) and ristocetin cofactor activity (vWF:RICO) were measured. Cardiovascular mortality was recorded over a median of five years...
July 20, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28721832/biomarkers-associated-with-bleeding-risk-in-the-setting-of-atrial-fibrillation
#2
Skevos Sideris, Stefanos Archontakis, George Latsios, George Lazaros, Konstantinos Toutouzas, Eleftherios Tsiamis, Manolis Vavuranakis, Charalampos Vlachopoulos, Konstantinos Gatzoulis, Konstantinos Tsioufis, Dimitris Tousoulis
Background Prevention of thromboembolic disease, mainly stroke, with oral anticoagulants remains a major therapeutic goal in patients with atrial fibrillation. Unfortunately, despite the high efficacy, anticoagulant therapy is associated with a significant risk of, frequently catastrophic, hemorrhagic complications. Among different clinical and laboratory parameters related to an increased risk of bleeding, several biological markers have been recognized and various risk scores for bleeding have been developed...
July 18, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28720509/the-neuropeptide-galanin-promotes-an-anti-thrombotic-phenotype-on-endocardial-endothelial-cells-from-heart-failure-patients
#3
Christina Tyrrell, Amanda Toyooka, Faiza Khan, Kent L Thornburg, James O Mudd, Wohaib Hasan
Thromboembolic complications are a significant cause of mortality and re-hospitalization in heart failure (HF) patients. One source of thrombi is the ventricular endocardial surface that becomes increasingly pro-thrombotic as HF progresses. Anticoagulation comes with bleeding risks so identifying therapeutic agents for improving cardiac endothelial health are of critical clinical importance. Endocardial endothelial cells are closely apposed to cardiac sympathetic nerves. In HF, cardiac sympathetic nerves are dysregulated and promote disease progression...
July 12, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28712423/anxiety-and-depression-in-patients-three-months-after-myocardial-infarction-association-with-markers-of-coagulation-and-the-relevance-of-age
#4
Franziska Geiser, Anne Sarah Urbach, Ursula Harbrecht, Rupert Conrad, Bernd Pötzsch, Nele Amann, Katharina Kiesewetter, Alexandra Sieke, Kyra Wolffs, Dirk Skowasch
OBJECTIVE: Anxiety and depression are associated with an activation of coagulation and an impairment of fibrinolysis, which may contribute to the increased cardiovascular risk associated with the two disorders. However, very few studies have examined the impact of psychological distress on coagulation factors in coronary artery disease patients. The aim of this study was to assess the correlation between anxiety/depression and factors of coagulation and fibrinolysis in patients who had suffered an acute MI three months prior...
August 2017: Journal of Psychosomatic Research
https://www.readbyqxmd.com/read/28709930/particle-image-velocimetry-study-of-the-celiac-trunk-hemodynamic-induced-by-continuous-flow-left-ventricular-assist-device
#5
Francesco Scardulla, Diego Bellavia, Leonardo D'Acquisto, Giuseppe M Raffa, Salvatore Pasta
Whereas left ventricular assist device (LVAD) is the gold-standard therapy for patients with heart failure, gastrointestinal bleeding is one of the most common complications. LVAD implantation may remarkably impact aortic hemodynamics so that experimental and computational flow analyses can be used to study the disease mechanisms. Here we present an experimentally-calibrated computational model of the celiac trunk hemodynamic of a LVAD-supported patient who experienced bleeding after device implantation. Specifically, both particle image velocimetry (PIV) and echocardiography were used to measure and compare flow distributions in each branch of a phantom model of the patient abdominal aorta...
July 12, 2017: Medical Engineering & Physics
https://www.readbyqxmd.com/read/28705716/occult-hepatitis-b-virus-infection-in-greek-patients-with-congenital-bleeding-disorders
#6
Agoritsa Varaklioti, Anna Kouramba, Panagiota Ioannidou, Olga Katsarou
Occult hepatitis B infection (OBI) is a form of chronic HBV infection characterized by low level HBV DNA, without detectable HBV surface antigen (HBsAg). OBI is frequently associated with the presence of anti-HBc and in some cases also with anti-HBs. Patients, who formerly received non-inactivated factor concentrates, can potentially be considered at high risk for OBI, especially since these patients usually are HIV or HCV co-infected. This study aimed to assess the prevalence of occult HBV infection in Greek patients with hereditary bleeding disorders...
July 10, 2017: Infection, Genetics and Evolution
https://www.readbyqxmd.com/read/28704418/the-coll%C3%AE-gen-iii-fibril-has-a-flexi-rod-structure-of-flexible-sequences-interspersed-with-rigid-bioactive-domains-including-two-with-hemostatic-roles
#7
J Des Parkin, James D San Antonio, Anton V Persikov, Hayat Dagher, Raymond Dalgleish, Shane T Jensen, Xavier Jeunemaitre, Judy Savige
Collagen III is critical to the integrity of blood vessels and distensible organs, and in hemostasis. Examination of the human collagen III interactome reveals a nearly identical structural arrangement and charge distribution pattern as for collagen I, with cell interaction domains, fibrillogenesis and enzyme cleavage domains, several major ligand-binding regions, and intermolecular crosslink sites at the same sites. These similarities allow heterotypic fibril formation with, and substitution by, collagen I in embryonic development and wound healing...
2017: PloS One
https://www.readbyqxmd.com/read/28702343/eb-virus-reactivation-triggers-thrombotic-thrombocytopenic-purpura-in-a-healthy-adult
#8
Satoko Oka, Masaharu Nohgawa
Thrombotic thrombocytopenic purpura (TTP) is rare but life-threatening disease, characterized typically by microangiopathic hemolytic anemia (MAHA), profound peripheral thrombocytopenia and severe deficiency in the von Willebrand factor-cleaving prortease ADAMTS13. It has been reported that acquired immune TTP is closely associated with human immunodeficiency virus infection and influenza infection or vaccination. However, it has not been reported to be associated with Epstein Barr Virus infection or reactivation...
2017: Leukemia Research Reports
https://www.readbyqxmd.com/read/28696025/a-novel-vwf-variant-associated-with-type-2-von-willebrand-disease-in-german-wirehaired-pointers-and-german-shorthaired-pointers
#9
M Vos-Loohuis, B A van Oost, C Dangel, I Langbein-Detsch, P A Leegwater
Von Willebrand disease (VWD), caused by deficiency of the von Willebrand factor (VWF), is the most common bleeding disorder in humans and dogs. The complete cDNA encoding VWF of a German Wirehaired Pointer with type 2 VWD was sequenced, and we found four variants that alter the amino acid sequence. These variants were: c.1657T>G corresponding to p.Trp553Gly; c.1777G>A (p.Glu593Lys); c.4937A>G (p.Asn1646Ser) and c.5544G>A (p.Met1848Ile). A haplotype of the c.1657G, c.1777A and c.4937G alleles co-segregated with the VWF antigen level in a four-generation pedigree with the disease...
August 2017: Animal Genetics
https://www.readbyqxmd.com/read/28693615/genome-analysis-of-clostridium-perfringens-isolates-from-healthy-and-necrotic-enteritis-infected-chickens-and-turkeys
#10
Troels Ronco, Marc Stegger, Kim Lee Ng, Berit Lilje, Ulrike Lyhs, Paal Skytt Andersen, Karl Pedersen
OBJECTIVE: Clostridium perfringens causes gastrointestinal diseases in both humans and domestic animals. Type A strains expressing the NetB toxin are the main cause of necrotic enteritis (NE) in chickens, which has remarkable impact on animal welfare and production economy in the international poultry industry. Three pathogenicity loci NELoc-1, -2 and -3 and a collagen adhesion gene cnaA have been found to be associated with NE in chickens, whereas the presence of these has not been investigated in diseased turkeys...
July 11, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28692141/a-discontinuous-autoinhibitory-module-masks-the-a1-domain-of-von-willebrand-factor
#11
Wei Deng, Yingchun Wang, Samuel A Druzak, John F Healey, Anum K Syed, Pete Lollar, Renhao Li
BACKGROUND: How von Willebrand factor (VWF) senses and responds to shear flow remains unclear. In the absence of shear VWF or its fragments can be induced to bind spontaneously to platelet GPIbα. Objectives To elucidate the auto-inhibition mechanism of VWF. METHODS: Hydrogen-deuterium exchange (HDX) of two recombinant VWF fragments expressed from baby hamster kidney cells were measured and compared. RESULTS: The shortA1 protein contains VWF residues 1261-1472 and binds GPIbα with a significantly higher affinity than the longA1 protein that contains VWF residues 1238-1472...
July 10, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28692107/whole-blood-ristocetin-activated-platelet-impedance-aggregometry-multiplate-for-the-rapid-detection-of-von-willebrand-disease
#12
David E Schmidt, Maria Bruzelius, Ammar Majeed, Jacob Odeberg, Margareta Holmström, Anna Ågren
Von Willebrand disease (VWD) is the most common bleeding disorder, but no bedside tests specific for Von Willebrand factor are available. The objective of this study was to evaluate the diagnostic accuracy of whole blood ristocetin-induced platelet aggregometry (WB-RIPA) in VWD. WB-RIPA was performed in VWD patients (n=100) and healthy controls (n=17) using the Multiplate® platelet impedance aggregometry platform. The diagnostic properties of the test were described as sensitivity/specificity, positive and negative predictive value, and ROC area under the curve (AUC)...
July 6, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28691669/spontaneous-chronic-subdural-hematoma-in-young-adult-the-role-of-missing-coagulation-facto
#13
M Dobran, M Iacoangeli, A R Scortichini, F Mancini, R Benigni, D Nasi, M Gladi, M Scerrati
AIM: Chronic subdural hematoma (CSDH) is typically in elderly and rarely in young people. To prevent complications and re-bleeding after surgical treatment of CSDH it is important to assess the risk factors as coagulation disorders especially in young patients (below 65 years) with no history of head trauma, alcohol abuse or anticoagulant therapy. PATIENTS AND METHODS: This study consists of 16 patients (12 males, 4 females) with age ranging from 27 to 59 years (median 48,25 years) operated for CSDH...
March 2017: Il Giornale di Chirurgia
https://www.readbyqxmd.com/read/28691218/safety-of-a-pasteurized-plasma-derived-factor-viii-and-von-willebrand-factor-concentrate-analysis-of-33-years-of-pharmacovigilance-data
#14
Peter Kouides, Kathrin Wawra-Hehenberger, Anna Sajan, Henry Mead, Toby Simon
BACKGROUND: Haemate-P/Humate-P (Humate-P) is a pasteurized human plasma-derived concentrate containing both Factor VIII and von Willebrand factor for treatment of hemophilia A and von Willebrand disease (VWD). STUDY DESIGN AND METHODS: We analyzed the safety of Humate-P based on more than 33 years of postmarketing pharmacovigilance data, representing an estimated exposure of approximately 25,000 patient-years. The analysis comprises reports of potential adverse drug reactions (ADRs) from all sources, reported as part of routine pharmacovigilance at CSL Behring...
July 10, 2017: Transfusion
https://www.readbyqxmd.com/read/28686158/diagnosis-and-management-of-patients-with-von-willebrand-s-disease-in-italy-an-expert-meeting-report
#15
EDITORIAL
Flora Peyvandi
No abstract text is available yet for this article.
May 26, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28679991/acquired-immune-mediated-von-willebrand-syndrome-accompanied-by-antiphospholipid-syndrome
#16
Nobuhiko Kobayashi, Yoshiyuki Ogawa, Kunio Yanagisawa, Takuma Ishizaki, Hideki Uchiumi, Nobuaki Suzuki, Tadashi Matsushita, Akitada Ichinose, Hiroshi Handa
Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings resembling those of congenital von Willebrand disease. AvWS usually occurs in association with a variety of underlying disorders, such as lymphoproliferative disease or cardiovascular disease, but autoimmune AvWS is very rare. We now describe the case of a 42-year-old woman with autoimmune AvWS with concurrent antiphospholipid syndrome (APS). The patient was suffering from epistaxis and menorrhagia from few years prior to referral...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28679486/anti-inflammatory-effects-of-active-commuting-and-leisure-time-exercise-in-overweight-and-obese-women-and-men-a-randomized-controlled-trial
#17
Anne Sofie Gram, Else-Marie Bladbjerg, Jonas Salling Quist, Martin Bæk Petersen, Mads Rosenkilde, Bente Stallknecht
BACKGROUND AND AIMS: Physical inactivity is linked to low-grade inflammation, endothelial dysfunction and cardiovascular disease. We aimed to determine effects of active commuting and leisure time exercise on markers of low-grade inflammation and endothelial function in overweight and obese women and men. METHODS: We randomized 130 younger (20-45 years), physically inactive, healthy, overweight and obese (BMI: 25-35 kg/m(2)) women and men recruited from the Copenhagen area, Denmark, to either 6 months of habitual lifestyle (CON, n = 18), active commuting (BIKE, n = 35), or leisure time exercise of moderate (MOD, ∼50% VO2peak, n = 39) or vigorous intensity (VIG, ∼70% VO2peak, n = 38)...
June 28, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28674874/a-protocol-for-the-preparation-of-cryoprecipitate-and-cryo-depleted-plasma-for-proteomic-studies
#18
Rosemary L Sparrow, Richard J Simpson, David W Greening
Cryoprecipitate is a concentrate of high-molecular-weight plasma proteins that precipitate when frozen plasma is slowly thawed at 1-6 °C. The concentrate contains factor VIII (antihemophilic factor), von Willebrand factor (vWF), fibrinogen, factor XIII, fibronectin, and small amounts of other plasma proteins. Clinical grade preparations of cryoprecipitate are mainly used to treat fibrinogen deficiency caused by acute bleeding or functional abnormalities of the fibrinogen protein. In the past, cryoprecipitate was used to treat von Willebrand disease and hemophilia A (factor VIII deficiency), but the availability of more highly purified coagulation factor concentrates or recombinant protein preparations has superseded the use of cryoprecipitate for these coagulopathies...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28674146/self-reported-sleep-duration-and-napping-cardiac-risk-factors-and-markers-of-subclinical-vascular-disease-cross-sectional-study-in-older-men
#19
Shahrzad Zonoozi, Sheena E Ramsay, Olia Papacosta, Lucy Lennon, Elizabeth A Ellins, Julian P J Halcox, Peter H Whincup, S Goya Wannamethee
STUDYOBJECTIVES: Daytime sleep has been associated with increased risk of cardiovascular disease and heart failure (HF), but the mechanisms remain unclear. We have investigated the association between daytime and night-time sleep patterns and cardiovascular risk markers in older adults including cardiac markers and subclinical markers of atherosclerosis (arterial stiffness and carotid intima-media thickness (CIMT)). METHODS: Cross-sectional study of 1722 surviving men aged 71-92 examined in 2010-2012 across 24 British towns from a prospective study initiated in 1978-1980...
July 2, 2017: BMJ Open
https://www.readbyqxmd.com/read/28670433/thalidomide-for-the-treatment-of-gastrointestinal-bleeding-due-to-angiodysplasia-in-a-patient-with-glanzmann-s-thrombasthenia
#20
Bruno K L Duarte, Sílvia M de Souza, Carolina Costa-Lima, Samuel S Medina, Margareth C Ozelo
Angiodysplasia is a frequent cause of persistent gastrointestinal (GI) hemorrhage in elderly patients. Although GI bleeding isn't the most common manifestation in patients with bleeding disorders, when present, it represents a challenging complication. We describe a 62-year-old patient with Glanzmann's thrombasthenia, who used thalidomide for severe and recurrent GI bleeding. For 6 months, the patient experienced temporary control of GI bleeding with thalidomide in a daily oral dose of 100 mg. The anti-angiogenic effects of thalidomide have recently been explored by several groups, particularly in the management of bleeding from angiodysplasia, including cases with von Willebrand disease...
June 1, 2017: Hematology Reports
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